Neonatal lupus erythematosus is a disease primarily characterized by c
ardiac and/or cutaneous involvement. Liver and haematological involvem
ent occur in a few cases and these are normally considered to be mild
and transient complications. The disease is suspected to be caused by
transplacental passage of maternal autoantibodies, primarily anti-Ro/S
SA and anti-La/SSB, from mothers with systemic lupus erythematosus. He
re we report a child with neonatal lupus erythematosus who died from s
evere liver and haematological disease when 2 d old. The baby had no c
ardiac or cutaneous involvement. The mother has had two subsequent pre
gnancies. In the first she had a boy without certain signs of neonatal
lupus erythematosus. In the next pregnancy the child developed congen
ital complete heart block and cutaneous lesions.